Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999. Human HO-1 deficiency has been observed to involve the endothelial cells more severely, resulting in hemolysis and disseminated intravascular coagulation. We report another case of human HO-1 deficiency in a young girl with congenital asplenia, who presented with severe hemolysis, inflammation, nephritis, which was refractory to therapy with corticosteroids, cyclophosphamide, and rituximab.
Summary
Guidelines for the use of polymerase chain reaction (PCR)‐based assays to aid the diagnosis of invasive aspergillosis (IA) in high‐risk haematology patients have not been formulated. We prospectively evaluated a nested PCR assay to detect Aspergillus in blood during 95 febrile neutropenic episodes, in patients with haematological malignancy and haematopoietic stem cell transplant (HSCT) recipients. PCR results were correlated with the diagnostic classification of the 2002 European Organisation for Research and Treatment of Cancer/Mycosis Study Group. When two‐positive results were used to define an episode as ‘PCR positive’, the sensitivity, specificity, positive‐predictive value and negative predictive value for ‘proven’/‘probable’ IA (n = 13) were 100%, 75·4%, 46·4% and 100%, respectively. Consecutive positive results occurred in 61·5% of these 13 episodes. Overall, PCR positivity preceded standard diagnosis by a mean of 14 d and the median time between positive results was shorter than that in other categories of IA. All 13 episodes occurred in the setting of allogeneic HSCT recipients and acute leukaemia. If ‘eligibility’ for antifungal therapy were based on two‐positive‐PCR tests, use of empiric treatment could have been reduced by up to 37%. The nested PCR assay is a practical screening test for excluding IA. Patients with consecutive positive results or intermittent‐positive results (within 14 d) warrant immediate investigations for IA and the initiation of antifungal therapy.
Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis.
The survival of children with cancer in India is inferior to that of children in high-income countries. The Indian Pediatric Hematology Oncology Group (IPHOG) held a series of online meetings via www.Cure4kids.org to identify barriers to cure and develop strategies to improve outcomes. Five major hurdles were identified: delayed diagnosis, abandonment, sepsis, lack of co-operative groups, and relapse. Development of regional networks like IPHOG has allowed rapid identification of local causes of treatment failure for children with cancer in India and identification of strategies likely to improve care and outcomes in the participating centers. Next steps will include interventions to raise community awareness of childhood cancer, promote early diagnosis and referral, and reduce abandonment and toxic death at each center. Starting of fellowship programs in pediatric hemato-oncology, short training programs for pediatricians, publishing outcome data, formation of parent and patient support groups, choosing the right and effective treatment protocol, and setting up of bone marrow transplant services are some of the effective steps taken in the last decade, which needs to be supported further.
S. P. Yadav et al. complex cytogenetics in 2, XO in 2, trisomy 8 in 1) disease. In the favorable-risk group, 4 died, 5 were lost to follow-up, and only 1 is alive. Among 5 patients with t(15; 17), only 2 opted for therapy. One died in induction with intracranial bleed and the other relapsed 1 year after finishing therapy. In the intermediate-risk group, 2 died during induction and 2 are alive and disease-free. In the high-risk group, 3 died, 2 were lost to follow-up, and 2 are alive with 1 disease-free.High mortality with intensive protocol, high relapse rates with less intensive protocols, and treatment abandonment are major barriers to improving outcome for pediatric AML in the developing world. Less intensive protocols with better efficacy are the need of the hour.
Declaration of interestPediatric Hematology and Oncology
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